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Results for “PULMONARY FIBROSIS”

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PubMed1992

Circulating intercellular adhesion molecule-1 (ICAM-1) antigen in sera of patients with idiopathic pulmonary fibrosis.

Intercellular adhesion molecule-1 (ICAM-1), a member of immunoglobulin supergene family with a five-domain structure, is known to play an important role in inflammatory diseases. An ELISA was developed using two MoAbs against human ICAM-1 in order to detect the soluble shedding ICAM-1 antigen in sera. We measured levels of circulating ICAM-1 antigen in sera of patients with idiopathic pulmonary fibrosis (IPF), pulmonary sarcoidosis, hypersensitive pneumonitis, bacterial and mycoplasmal pneumonia, and inflammatory diseases of other organs. The results clearly demonstrated that IPF had significantly high levels of circulating ICAM-1 in sera as compared with other disorders or normal controls. Moreover, immunohistochemical analysis with MoAb against human ICAM-1 disclosed that in IPF, the expression of ICAM-1 was intensively enhanced on alveolar epithelial cells. These results suggest that ICAM-1 may contribute to the pathogenesis of IPF.

Adult↗

PubMed2002

Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis.

OBJECTIVE: We attempted to evaluate the feasibility (therapeutic efficacy, tolerance, and clinical courses after treatment) of pirfenidone, an anti-fibrotic drug for patients with chronic pulmonary fibroses such as idiopathic pulmonary fibrosis (IPF). METHODS: Open-label one-year treatment for compassionate-use. PATIENTS OR MATERIALS: Oral pirfenidone (40 mg/kg body weight) was administered to 8 patients with advanced IPF and 2 with interstitial pneumonia associated with diffuse systemic sclerosis. The plasma concentration of the drug was serially followed. Radiographic scores, pulmonary functions, and arterial blood oxygen pressure were compared at three time points: at one-year before treatment, at the time of entry, and at one-year after entry. RESULTS: While pirfenidone did not show a definite therapeutic effect on overall survival (2 years after entry). During one-year treatment, there was no significant deterioration in terms of chest radiographic scores and arterial oxygen pressure, and the drug was well tolerated with minimal adverse effects within the ranges observed in this study, plasma pirfenidone concentrations did not seem to relate to the appearance of adverse effects or differences in therapeutic effects. CONCLUSION: The feasibility of Pirfenidone as a therapeutic drug was confirmed for patients with advanced pulmonary fibrosis.

Aged↗

PubMed1983

Pulmonary fibrosis subsequent to high doses of CCNU for chronic myeloid leukemia.

Two patients treated for chronic myeloid leukemia with high doses of CCNU (1100 mg/m2 and 1240 mg/m2, respectively) developed a fatal pulmonary fibrosis. This side effect has never been reported for this nitrosourea but only for BCNU and methyl-CCNU. The responsibility of CCNU in the pathogenesis of pulmonary fibrosis seems very likely. The possibility that the underlying disease or other chemotherapeutic agents may increase the risk of pulmonary toxicity can, however, be discussed. In addition, 13 other adults treated for chronic myeloid leukemia with various doses of CCNU were reviewed. No respiratory symptoms appeared in the ten patients who were given less than 950 mg/m2. Three patients who received more than 1100 mg/m2 developed pulmonary symptoms with the same clinical and radiologic pattern as in the two cases with pathologic documents, and two of them died from acute respiratory failure. Although lung specimens were lacking from these three patients, it is suggested that the pulmonary toxicity of CCNU may be dose-related.

Adult↗

PubMed1981

[A documented case of typical Hamman-Rich interstitial pulmonary fibrosis].

Following a brief mention of the classification of Hammer-Rich disease within idiopathic diffuse pulmonary fibrosis, the main clinical, radiological and laboratory features of these disease are outlined in brief. A personal case, subsequently controlled neroptically and histologically, is then described. From comparison between reported data and findings in the personal case, it is concluded that it was a typical, rare case (less than 150 others have so far been reported in all world literature) of Hamman-Rich disease. Other diagnostic hypotheses can be rejected because they are less correspondent to the typical picture and for various other considerations.

Cyanosis↗

PubMed1982

Differential cellular analysis of bronchoalveolar lavage fluid obtained at various stages during the development of bleomycin-induced pulmonary fibrosis in the rat.

The purpose of this study was to analyze the cellular components of bronchoalveolar lavage fluid throughout the development of bleomycin-induced pulmonary fibrosis in the rat. Animals were killed and lavaged at various times after the administration of a single intratracheal injection of bleomycin. The results demonstrate that a significant influx of inflammatory cells appear in the lavage fluid as early as Day 1 after bleomycin treatment. Polymorphonuclear leukocytes are the first cells to appear and significant concentrations persist for as long as 1 month after bleomycin treatment. There is a very transient yet significant influx of eosinophils on Day 7 after bleomycin treatment. Lymphocytes are present from 3 to 14 days after bleomycin treatment; greater than 97% are T-cells and less than 3% are B-cells. There is a 1:1 ratio of W3/25+ cells (helper cell activity) to OX8+ cells (suppressor cell activity) comprising the lymphocyte population. The blood and lymphoid tissue of these animals contain a normal 2:1 ratio of these subsets. The data demonstrate that specific T-cell populations are present in the air spaces of the lung in response to bleomycin-induced pulmonary fibrosis in this model.

Animals↗

PubMed2004

Heterogeneous increase in CD34-positive alveolar capillaries in idiopathic pulmonary fibrosis.

To elucidate the apparent contradictions in vascular remodeling in the lungs of patients with idiopathic pulmonary fibrosis, we evaluated alveolar vascularity in relation to the various degrees of fibrosis in surgically biopsied lungs of usual interstitial pneumonia. Alveolar capillary endothelial cells were intensely immunoreactive with CD34 but not with von Willebrand factor. Vascular density, that is, the relative ratio of capillary area to total area of alveolar walls, was significantly higher at low grades of fibrosis than in control lungs, whereas vascular density gradually decreased as the degree of fibrosis increased and was lower than that of control lungs in the most extensively fibrotic lesions. No vessels were observed inside fibroblastic foci. The potent angiogenic factors vascular endothelial growth factor and interleukin-8 were abundantly produced by capillary endothelial cells and alveolar epithelial cells in highly vascularized alveolar walls. In contrast, venules with CD34-negative but von Willebrand factor-positive endothelial cells localized in the center of the fibrotic lesions were slightly increased and identified as postcapillary venules by three-dimensional reconstructed images. These results indicate the presence of heterogeneous vascular remodeling in usual interstitial pneumonia.

Antigens, CD34↗

PubMed1989

Primary sclerosing cholangitis and idiopathic pulmonary fibrosis: a case report.

Primary sclerosing cholangitis (PSC) and idiopathic pulmonary fibrosis (IPF) were each separately described in association with a variety of chronic idiopathic inflammatory diseases such as retroperitoneal fibrosis, Sicca complex or Riedel's thyroiditis. We report a case which may be the first description of the association between PSC and IPF. The physiopathogenic implications of such an association are discussed.

Biopsy↗

PubMed1983

Lavage type III procollagen N-terminal peptides in human pulmonary fibrosis and sarcoidosis.

Levels of type III procollagen N-terminal peptide were immunoassayed in sera and bronchoalveolar lavage fluids from healthy volunteers and patients with idiopathic pulmonary fibrosis or sarcoidosis. Peptide levels normalized to protein were higher than corresponding serum levels in all groups, suggesting that the lung is a producer of this peptide. Levels were significantly elevated in both patient groups, suggesting increased rates of type III collagen synthesis. Values were highest in idiopathic pulmonary fibrosis samples. Correlation of peptide levels with clinical severity of disease and lavage cell profiles was poor, perhaps because the latter reflects aspects of the disease process other than that stage in which type III collagen synthesis is altered. Lavage analyses of procollagen peptides may provide an important index of altered collagen synthesis in interstitial lung disease.

Amino Acid Sequence↗

PubMed1993

[Histiocytic lymphoma and pulmonary fibrosis in a female patient with essential thrombocythemia].

The case of a patient with the diagnosis of essential thrombocythemia is presented. Following treatment with melphalan during three years the patient presented clinical and radiologic data of pulmonary fibrosis. Thoracotomy with lung biopsy histologically proving fibrosis was performed. The patient developed a true histiocytic lymphoma afterwards. The rarity of pulmonary fibrosis induced by melphalan and the exceptional association of essential thrombocythemia and histiocytic lymphoma is emphasized. The characteristics of the latter disease, diagnostic difficulties and possible treatment are commented upon.

Adult↗

PubMed1993

[Phospholipid components in BALFS from bleomycin induced pulmonary fibrosis in rats].

Qualitative and quantitative analysis was performed on 5 components of phospholipids (PL) isolated from bronchoalveolar lavage fluid (BALF) during the development of bleomycin induced pulmonary fibrosis in rats with high-performance liquid chromatography (HPLC). The measurement of cells and protein in BALF, the pathologic and morphometric observation with light and electronic microscope were also done. Results indicated that the changes both in BALF cells and in lung histopathology were similar, the amount of protein in BALF increased, the total amount of PL in BALF increased more than 1.8-fold over control animals at days 28 after administration of Bleomycin, phosphatidylglycerol (PG) decreased with time, phosphatidylinositol (PI) and phosphatidylethanolamine (PE) increased, PG/PI ratio decreased, the PG/PI ratio could be used as a marker for pulmonary fibrosis.

Animals↗

PubMed1980

Evaluation of antifibrotic drugs in bleomycin-induced pulmonary fibrosis in hamsters.

Endotracheal administration of bleomycin to hamsters causes severe pulmonary fibrosis. We have examined the utility of this response as a model for the screening of pulmonary antifibrotic agents. The time course of collagen synthesis after bleomycin administration was examined in neutral salt soluble and insoluble fractions by in vitro incubation of minced lung with [14C] proline. Collagen synthesis increased to approximately 250% above control in both neutral salt soluble and insoluble collagen fractions by day 6 after bleomycin. Noncollagenous protein synthesis was also increased but to a lesser amount. The early rise in collagen synthesis leads to accumulation of collagen that can be biochemically quantitated within 1 week. This time course is advantageous for short-term testing of antifibrotic agents. In the present study, beta-aminopropionitrile, D-penicillamine, and p-aminobenzoic acid were examined. All three agents were found to reduce significantly the accumulation of neutral salt insoluble collagen in bleomycin-treated animals.

4-Aminobenzoic Acid↗

PubMed1986

[Pneumonitis with fatal pulmonary fibrosis (Hamman-Rich syndrome) due to parathion-(E-605-) poisoning].

A patient with chronic Parathione (E 605) poisoning was observed over a period of 55 days. During that time he developed progressive changes, which were identical to those of progressive idiopathic pulmonary fibrosis. The rapid development of an alveolitis, followed by a lethal pulmonary fibrosis, differed in no way, macroscopically nor microscopically, from the lung changes in paraquat poisoning (paraquat lung). The radiological course has been correlated with the clinical and post mortem findings.

Humans↗

PubMed2002

Relaxin deficiency in mice is associated with an age-related progression of pulmonary fibrosis.

Relaxin (RLX) is a peptide hormone with known antifibrotic properties. However, its significance in the lung and its role as a therapeutic agent against diseases characterized by pulmonary fibrosis are yet to be established. In this study, we examined age-related structural and functional changes in the lung of relaxin-deficient mice. Lung tissues of male and female RLX knockout (-/-) and RLX wild-type (+/+) mice at various ages were analyzed for changes in collagen expression and content. We demonstrate an age-related progression of lung fibrosis in RLX -/- mice with significantly increased tissue wet weight, collagen content and concentration, alveolar congestion, and bronchiole epithelium thickening. The increased fibrosis was associated with significantly altered peak expiratory flow and lung recoil (lung function) in RLX -/- mice. Treatment of RLX -/- mice with relaxin in early and developed stages of fibrosis resulted in the reversal of collagen deposition. Organ bath studies showed that precontracted lung strips relaxed in the presence of relaxin. Together, these data indicate that relaxin may provide a means to regulate excessive collagen deposition in diseased states characterized by pulmonary fibrosis.

Aging↗

PubMed2004

Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment.

Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal pulmonary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomography demonstrates evidence of fibrosis and lung remodelling such as honeycomb cysts and traction bronchiectasis. There is no known effective treatment for IPF, but lung transplantation improves survival.

Adult↗

PubMed1981

Bleomycin-induced diffuse interstitial pulmonary fibrosis in baboons. II. Further studies on connective tissue changes.

Pulmonary fibrosis induced by bleomycin is associated with accumulation of collagen and elastin in the lungs. The excess connective tissue proteins persist despite resolution of inflammation after cessation of treatment. In the present study, mild lung injury was produced in 9 juvenile baboons by twice-weekly injections of bleomycin to a total dose of 66 units/kg. Treated animals showed losses in body weight, lung volume, and diffusing capacity. Right middle lobectomies were performed in 3 animals shortly after cessation of bleomycin. Only minimal histologic changes were present, but lobar connective tissue protein concentrations and the rate of collagen synthesis were increased. Biopsies obtained in 3 additional animals 3 months later revealed similar changes. All animals were killed 6 months after cessation of treatment. Mild fibrosis was present, and lobar contents of collagen and elastin, as well as synthetic rates of collagen and elastin, remained elevated. Accumulation of lung connective tissue proteins in this model was associated with increased rates of synthesis that persisted after discontinuance of the drug.

Animals↗

PubMed1990

[Eye disease in pulmonary fibrosis].

The author describes a case of ocular affection in pulmonary fibrosis with an obscure aetiology and subsequent secondary polyglobulia. In addition to cyanosis of the mucosae and conjunctive the authors detected also cyanosis of the retina of both eyes and thrombosis of the central retinal vein on the right and prethrombosis on the left. The author gives an account of the differential diagnosis of diseases with a similar ophthalmological finding.

Eye Diseases↗

PubMed1991

Cells containing factor XIIIa and pulmonary fibrosis induced by bleomycin.

To show the clinical importance of cells containing FXIIIa in pulmonary fibrosis induced by bleomycin, the distributions of FXIIIa and collagenous components were investigated immunohistochemically in both normal lung tissues and those affected by bleomycin. In the normal tissues FXIIIa-containing cells were sparse, but they were numerous in the pulmonary fibrotic tissues, especially in the subpleural area and around the blood vessels of alveolar septa, where slight to moderate fibrosis was seen, and in the intra-alveolar fibrinous exudate. In the collagenous scar-like areas, however, these cells were fewer in number and their FXIIIa expression was depleted. These findings suggest that cells containing FXIIIa have an important role in the development of pulmonary fibrosis induced by bleomycin.

Adult↗

PubMed1996

Inheritance of susceptibility to bleomycin-induced pulmonary fibrosis in the mouse.

Based on the range of patient responses to treatment, and on animal studies, it is hypothesized that individual variation in sensitivity to bleomycin-induced pulmonary fibrosis is controlled genetically. A genetic model has been developed by (a) establishing a distinct difference in bleomycin-induced lung damage in two inbred strains of mice [parental generation: C57BL/6J (fibrosis-prone phenotype) and C3Hf/Kam (fibrosis-resistant phenotype)] and (b) characterizing inheritance of the fibrosing phenotype in the F1 (first filial) and F2 (F1 intercross; second filial) generations derived from the parental strains. Male mice received 100 mg/kg and female mice 125 mg/kg of bleomycin via s.c. osmotic minipump. The animals were sacrificed 8 weeks after treatment or when their breathing rate indicated respiratory distress. The percentage of lung with fibrosis for each mouse was quantified with image analysis of a histological section of the left lung. The mean percentage of fibrosis for the C57BL/6J males was 8.4 +/- 0.8% (SE) and 4.4 +/- 0.8% for females, and the C3Hf/Kam mice of either sex did not present the fibrosing lesion (mean score, 0%). Significant difference (P = 6 x 10(-6)) was measured in percentage of fibrosis between the two strains of F1 males, but not F1 females (P = 0.38), suggesting the presence of an X-linked factor associated with the fibrosing phenotype. From an ANOVA the X-linked factor is estimated to contribute 19% of the fibrosis phenotype. A genetic model of two or three loci controlling the fibrosing phenotype is proposed from the data of the parental, F1, and F2 generations. The mouse model demonstrates that susceptibility to bleomycin-induced pulmonary fibrosis is a heritable trait controlled by a few genetic loci.

Animals↗